Whenever a primary diagnosis of bone or soft tissue sarcoma is made outside the context of a specialist sarcoma unit, wherever possible, referral to an expert pathologist (within a specialist sarcoma unit) for review of the diagnosis and grade should be undertaken before definitive management is instituted.

Patients with suspected sarcoma to be referred to a specialist sarcoma unit prior to diagnosis in order to reduce the rates of incomplete excision, reoperation, local recurrence and to improve survival.

In patients with non-metastatic truncal sarcomas, adding radiotherapy to surgery is appropriate to further improve local control. When offered, pre-operative radiotherapy is preferable to post-operative radiotherapy.

In patients with non-metastatic retroperitoneal sarcomas, adding radiotherapy to surgery is appropriate to further improve local control. When offered, pre-operative radiotherapy is preferable to post-operative radiotherapy.

It maybe reasonable to consider IMRT for patients with retroperitoneal and extremity/truncal sarcomas as adjuvant to surgery, if resource permits, for potential advantages in reduction of radiation dose to normal tissues.

D

Reconstruction using the patients own resected bone (previously bearing the sarcoma) fragment after a large extra-corporeal dose of radiation is a possible option reported to have satisfactory to good functional outcomes.

D

Particle beam therapy appears to offer good local control with acceptable toxicity.

D

Brachytherapy (as an alternate or as a boost to external beam radiation) improves local control over surgery alone for high grade sarcomas for the limb and trunk.

B

IORT boost to external radiation could be considered in combination with surgery for management of retroperitoneal sarcomas.

B

Surgery

Isolated limb perfusion should be considered in patients with extensive soft tissue sarcoma where there is doubt whether limb salvage surgery can be achieved. Decision should be made in the setting of a multidisciplinary team.

C

Soft tissue sarcomas initially excised with residual disease and/or positive margins will require re-excision, preferably in a specialist sarcoma unit. These tumours should be re-excised with wide margins and usually require adjuvant radiotherapy.

C

Grade 1 Chondrosarcoma can be safely managed with intralesional excision with cementation. Distinction between this and other grades requires correlation of clinical and radiological features.

C

Retroperitoneal sarcomas are best managed in a specialised tumour centre by a multidisciplinary unit.

C

Limb salvage surgery is an acceptable treatment in the management of osteosarcoma.

C

Pre-operative radiation therapy may allow preservation of vital structures without compromising local control.

C

It is important that wide surgical margin is achieved to prevent local recurrence and poor survival outcomes.

B

Pre or post-operative radiation therapy should be considered in the management of soft tissue sarcoma. Decision should be made in the setting of a multidisciplinary team.

A

Musculoskeletal tumours are best managed in a specialist sarcoma unit by a multidisciplinary team.

C

Practice point(s)

Any lump greater than 5 cm or deep to the deep fascia should be considered a sarcoma until proven otherwise.

Persistent and unremitting pain, not responsive to oral analgesics and nocturnal in occurrence should stimulate investigation for a bone tumour.

Complete imaging (anatomic and functional including XR, CT, MRI, nuclear scan) should be undertaken of a bone and soft tissue tumour prior to surgical manipulation.

Biopsy should be performed under image guidance to determine the track of the biopsy, and the target of the biopsy to confirm representativeness. Computed tomographic guidance is recommended. Biopsy should be performed after all imaging modalities have been completed to minimise the impact of biopsy induced image artifact.

Local recurrence is related to the adequacy of surgical margins. Wide surgical margins should be employed for bone and soft tissue sarcomas except when close margins are planned and adjuvant radiotherapy/chemotherapy is employed.

Tissues of different resistance to tumour invasion that surround a tumour may be used to calculate the quality of surgical margins. In this way, more careful planning of surgical margins may be undertaken when contemplating limb-sparing surgery.

Combination therapy is required to adequately manage bone and soft tissue sarcomas. Radiotherapy and wide margin surgery are used for soft tissue sarcomas. Chemotherapy and wide margin surgery are used for bone sarcomas.

Radiotherapy is recommended for low grade soft tissue sarcomas particularly if these tumours are large and excised with marginal margins.

Provision of education and psychological support is an important component in holistic care of the sarcoma patient.

C

The decisions for reconstruction of skeletal elements are ideally made at a specialist sarcoma unit.

D

When restoration of vascularity to a limb is required following sarcoma resection, prioritise arterial reconstruction and consider the need for venous reconstruction.

D

Sarcomas are better managed in a specialist sarcoma unit with planning of primary resection, reconstruction and timing of radiotherapy (where required) for optimal outcome.

D

Practice point(s)

The nature of reconstruction of defects following sarcoma resection is often complex due to the required size of resection, likelihood of need for perioperative radiotherapy with associated surgical challenges, and variation in involved tissue types. Specialist Multidisciplinary Team management is advised for all cases for optimal outcome.

Optimisation of general patient factors, both physical (including diabetic control, nutrition, minimising smoking and avoiding preventable perioperative morbidity) and psychological, will provide benefits to patient outcome. Patient education regarding the disease process and treatment options is also important in achieving the best holistic outcome.

Radiotherapy (in any form) reduces vascularity and impairs wound healing. Reconstructive options are affected by choice and timing of radiotherapy. A treatment plan for each case should be discussed at commencement of treatment to determine best timing and choice of surgical resection, surgical reconstruction and radiotherapy. This will allow best outcome with minimisation of surgical-related and radiotherapy-related morbidity.

When limb-preserving surgery is undertaken, care should be taken to reconstruct all resected tissues. This includes skeletal stability in bony reconstruction, reconstruction of neurovascular structures and functional muscle groups, and overlying soft tissue coverage.

In all resection defects requiring soft tissue coverage, vascularised tissue is the preferred reconstruction. This may be in the form of locoregional flap transfer, or free flap tissue transfer with reconstruction of the tissue vascularity using micro-surgical anastamoses of blood vessels. This enables best healing of underlying structures, reduces infection and other complication risks relating to skeletal implants, and provides greatest resilience to radiotherapy.

Restoration of function is the priority in reconstruction of the bony skeleton. Many options are available for reconstruction in metadiaphyseal areas, with preference for biological reconstruction where possible. Endoprosthetic reconstruction is commonly used in periarticular reconstruction.

Limb salvage procedures result in better functional outcomes, but do not necessarily result in greater quality of life.

Regular clinical examination is part of routine surveillance for local recurrence.

D

High risk patients in whom pulmonary metastasectomy would be considered, are advised to undergo three to six month CT chest until five years.

D

Practice point(s)

Where the primary site is difficult to examine, for example the retroperitoneum or following complex/flap reconstructions routine imaging may be appropriate.

Follow-up intervals recommended in current multinational guidelines are each three to four months in years one and two after diagnosis, six monthly in years three to four and annual thereafter.

Late metastases may occur >10 years after diagnosis and there is no universally accepted stopping point for tumour surveillance. By contrast, the incidence of late effects of treatment increases with time.

For patients enrolled in clinical trials, the above recommendations may vary in accordance with the follow-up protocols of these trials.

For patients considered suitable for pulmonary metastasectomy, low dose protocol non- contrast CT chest is the modality of choice for pulmonary surveillance.

Levels of evidence and grades for recommendations

The following table provides a list of the evidence-based recommendations detailed in the content of each topic question. The table below provides details on the highest level of evidence identified to support each recommendation (I-IV). The Summary of Recommendations table includes the grade for each recommendation (A-D). The key references that underpin the recommendation are provided in the last column. Individual levels of evidence can be found in the Evidence Summaries for each recommendation in each question.

Each recommendation was assigned a grade by the expert working group taking into account the volume, consistency, generalisability, applicability and clinical impact of the body of evidence supporting each recommendation.
When no Level I or II evidence was available and in some areas, in particular where there was insufficient evidence in the literature to make a specific evidence-based recommendation, but also strong and unanimous expert opinion amongst the working group members about both the advisability of making a clinically relevant statement and its content, recommended best practice points were generated. Thus, the practice points relate to the evidence in each question, but are more expert opinion-based than evidence-based. These can be identified throughout the guidelines with the following: Practice point (PP).

Grade of recommendation

Description

A

Body of evidence can be trusted to guide practice

B

Body of evidence can be trusted to guide practice in most situations

C

Body of evidence provides some support for recommendation(s) but care should be taken in its application

D

Body of evidence is weak and recommendation must be applied with caution

PP

(practice point)

Where no good-quality evidence is available but there is consensus among Guideline committee members, consensus-based guidance points are given, these are called "Practice points"